LEFT MAIN CORONARY-ARTERY ATRESIA - LITERATURE-REVIEW AND THERAPEUTICCONSIDERATIONS

Objective: Left main coronary artery atresia is a rare coronary anomal y in which there is no left coronary ostium, the proximal left main tr unk ends blindly, blood flows from the right coronary artery to the le ft via small collateral arteries and retrogradely in at least one of t he left-sided arteries. Since published case reports are few and rathe r scattered, no comprehensive information about this uncommon anomaly is available. Methods: A thorough search for published cases of left m ain coronary atresia was performed in the major medical journals by el ectronic (MEDLINE and INTERNET) and hand-scanning. Results: The author s found 28 cases of left main atresia (including two from their own ex perience), 15 of which were pediatric; five of these cases had associa ted cardiac anomalies. While pediatric patients were usually overtly s ymptomatic early in their life (syncope, dyspnea, sudden death, failur e to thrive, infarction, ventricular tachycardia), adult patients bega n showing symptoms (angina, dyspnea, sudden death) only at an advanced age; associated coronary atherosclerosis seemed to be uncommon, thoug h (2/13 adult patients: 15%). We know of only one asymptomatic patient , a 76-year old lady who had died of unrelated causes; in her case lef t main atresia was an unforeseen autopsy finding. Four untreated patie nts had died suddenly; most of the others were highly symptomatic and required surgical therapy, usually as direct coronary artery revascula rization via one or more saphenous vein or mammary artery grafts to th e left-sided arteries; all revascularized patients were reported to be alive and well; in one pediatric case the left main coronary artery w as reconstructed using an aortic wall baffle, with a good result. In c ontrast, the outcome of patients who did not receive revascularization has been poor. Conclusion: In light of the favorable results obtained by surgical therapy, the authors endorse prompt coronary artery revas cularization for all patients with left main coronary artery atresia. (C) 1997 Elsevier Science B.V.